Trait
Organic aciduria
Excretion of non-amino organic acids in urine.
Summary
Reference genomeGRCh38hg38
Positions on gene and variant pages follow this choice, in this browser.
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Get my Genome Test16 genes linked to Organic aciduria
Changes in these genes cause rare conditions in which Organic aciduria is seen. Most people with this trait have none of those conditions, and a gene on this list says nothing about anyone's own genome.
| Gene | How often it is seen | Conditions |
|---|---|---|
| ABCB7ATP binding cassette subfamily B member 7 | Always | In 2 conditions |
| BTDbiotinidase | Very often (80–99%) | In 2 conditions |
| MCCC1methylcrotonyl-CoA carboxylase subunit 1 | Very often (80–99%) | In 1 condition |
| MCCC2methylcrotonyl-CoA carboxylase subunit 2 | Very often (80–99%) | In 2 conditions |
| PCCApropionyl-CoA carboxylase subunit alpha | Very often (80–99%) | In 1 condition |
| PCCBpropionyl-CoA carboxylase subunit beta | Very often (80–99%) | In 1 condition |
| SLC25A19solute carrier family 25 member 19 | Very often (80–99%) | In 1 condition |
| HLCSholocarboxylase synthetase | Often (30–79%) | In 2 conditions |
| NADK2NAD kinase 2, mitochondrial | Often (30–79%) | In 1 condition |
| SLC25A4solute carrier family 25 member 4 | Often (30–79%) | In 1 condition |
| POLRMTRNA polymerase mitochondrial | Sometimes (5–29%) | In 1 condition |
| SLC52A2solute carrier family 52 member 2 | Sometimes (5–29%) | In 1 condition |
| FLAD1flavin adenine dinucleotide synthetase 1 | Not recorded | In 1 condition |
| GATMglycine amidinotransferase | Not recorded | In 1 condition |
| HMGCL3-hydroxy-3-methylglutaryl-CoA lyase | Not recorded | In 1 condition |
| MPC1mitochondrial pyruvate carrier 1 | Not recorded | In 1 condition |
How often is among people with one of the gene's conditions, the most often across them, as the Human Phenotype Ontology records it. These pages describe traits and the research behind them; they are not a diagnosis, and a doctor or genetic counsellor is the person to ask about symptoms.
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Where this comes from
- The Human Phenotype Ontology, release 2026-09-01: the trait, its names, its definition and the genes linked to it. Find out more at hpo.jax.org.
- Data release 2026.10.16-c6eea6bf.
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